Occasionally, readers submit questions regarding "late stage" or "end stage" MS. People want to know about "stage 4" MS, advanced MS or MS at the end of life. These questions are often difficult to answer, not just because of the sensitive nature of the subject, but due to the use of nonstandard terminology.
What are the types of MS?
Almost two decades ago, the National MS Society asked specialists in MS patient care and research to help develop a common language to use when discussing disease categories and characteristics. Four types of MS were defined as relapsing-remitting MS (RRMS), secondary progressive MS (SPMS), primary progressive MS (PPMS), and primary relapsing MS (PRMS).
RRMS is the most common type of MS affecting approximately 85% of MS patients at time of diagnosis. RRMS is characterized by episodes of distinct flare-ups (relapses, exacerbations) of acute worsening of neurological function. With RRMS, relapses are followed by recovery periods as partial or complete remissions. RRMS patients may experience residual symptoms between relapses, but they do not typically experience chronic worsening (progression) of the disease between relapses.
PPMS is relatively rare, affecting approximately 10% of MS patients at time of diagnosis. PPMS is characterized by a near continuous worsening from disease onset with occasional plateaus or minor improvements, while the rate of disease progression may vary over time. Patients with PPMS do not have distinct relapses or remissions.
PRMS is also relatively rare, affecting approximately 5% of MS patients. Like PPMS, primary-relapsing MS is characterized by a steady worsening of disease while patients experience the occasional relapse. The main difference between RRMS and PRMS is chronic worsening or disease progression between relapses. PRMS may have been (mis)diagnosed as PPMS until a relapse occurs which requires a change in diagnosis.
SPMS is the only type of MS which can be seen as a "next stage" of the disease progression. Patients with SPMS began their MS journey as RRMS patients, whether or not they were diagnosed appropriately at that time. The National MS Society states that "if left untreated, 50% of people with relapsing-remitting MS develop this form of the disease within 10 years of initial diagnosis." SPMS is characterized by a chronic steady worsening of the disease, with or without occasional relapses, incomplete remissions or plateaus in progression. The difference between SPMS and PRMS appears to be in how the person’s MS journey began.
What are the stages of MS?
One way to view this question is to consider a person’s experience living with MS. The first stage may include the recognition of unexplained symptoms which initially send a person to the doctor looking for answers, followed by the diagnosis itself. The second stage might encompass a person’s experience in learning to live with the disease, while adapting to the challenges MS places in the road. The next stage could be evidenced as the patient accumulates moderate disability.
Patients with advanced MS accumulate severe disability and often become dependent upon others for help with activities of daily living (ADL), such as feeding themselves, bathing, dressing, grooming, or homemaking. The final stage, or end stage MS, arises when severe disability and complications of MS create serious threats to a person’s life. When family members and caregivers ask questions regarding "end stage" MS, I presume (perhaps incorrectly) that they are really asking palliative or end-of-life questions.
[Edited to add: After a brief discussion with a friend whose wife has had MS for 27 years and who has a disability score of EDSS 8.5, I want to emphasize that a person can live with the more advanced stages of MS for many, many years. As Patrick said, “‘End stage’ sounds final but actually it can be the longest stage.”]
The National MS Society has published a number of booklets for patients, family members, and medical professionals regarding more advanced MS and end of life considerations. For more information, please read:
Caring for Loved Ones with Advanced MS: A Guide for Families (pdf) by Dorothy E. Northrop and Debra Frankel
Opening Doors: The Palliative Care Continuum in Multiple Sclerosis (pdf) by Marion Brandis, Nancy Clayton Reitman, David Gruenewald, and Maura Del Bene
Talking about Palliative Care, Hospice, and Dying (pdf) by Marion Brandis and Nancy Clayton Reitman
What are the phases of MS?
Beyond the scope of the present discussion, the phases of MS may also be described in terms of radiological findings. Early MS is characterized more by inflammation rather than later MS during which brain atrophy takes a prominent role. Changes in the immune system also occur over time as MS evolves. Researchers are also collecting evidence that disability progression in MS is a two-phase process (Leray 2010).
With the various ways in which simple words - stage, phase, type - are used in discussions surrounding MS, no wonder there is confusion when a doctor might say that your mother/sister/father/child has reached "stage 4" MS, for example. Anytime questions arise regarding aspects of your disease or that of your loved one, please ask the treating neurologist to explain the meaning and implication of what he/she says. Each person’s experience with MS is unique and specific needs may differ greatly from generalities.
Brandis M, Reitman NC. Talking about Palliative Care, Hospice, and Dying. © 2009 National Multiple Sclerosis Society
Brandis M, Reitman NC, Gruenewald D, Del Bene M. Opening Doors: The Palliative Care Continuum in Multiple Sclerosis. © 2008 National Multiple Sclerosis Society
Canadian Virtual Hospice. What can be expected with end-stage multiple sclerosis? Accessed online July 29, 2012.
Inglese M. Multiple Sclerosis: New Insights and Trends. AJNR May 2006; 27:954-957.
Leray E, et al. Evidence for a two-stage disability progression in multiple sclerosis. Brain 2010; 133(7):1900-1913. doi: 10.1093/brain/awq076 First published online: April 27, 2010
Northrop DE, Frankel D. Caring for Loved Ones with Advanced MS: A Guide for Families. © 2007 National Multiple Sclerosis Society
Teasell RW. Managing advanced multiple sclerosis. Can Fam Physician 1993 May; 39:1127-30, 1133-4, 1137-41.
Thompson AJ, et al. Long Term Management of Multiple Sclerosis. MS Management Apr 1994; 1(1).
Lisa Emrich is a patient advocate, accomplished speaker, author of the award-winning blog Brass and Ivory: Life with MS and RA, and founder of the Carnival of MS Bloggers. Lisa uses her experience to educate patients, raise disease awareness, encourage self-advocacy, and support patient-centered research. Lisa frequently works with non-profit organizations and has brought the patient voice to health care conferences and meetings worldwide. Follow Lisa on Facebook, Twitter, and Pinterest.